Vertebrobasilar Dolichoectasia, Hypoplastic Third Ventricle, and Related Biventricular Hydrocephalus: Case Report and Review of the Literature.

ABSTRACT

BACKGROUND: Vertebrobasilar dolichoectasia (VBDE) is defined as a symptomatic dilatation and tortuosity of the vertebrobasilar arteries. The risk of hydrocephalus development is due to direct compression of the third ventricle outflow or brainstem compression and related aqueduct stenosis. We present an uncommon case of a patient with symptomatic VBDE with the uniqueness of a hypoplastic third ventricle associated with biventricular hydrocephalus. A literature review concerning diagnosis and management of patients affected by biventricular hydrocephalus caused by VBDE was also performed. CASE ILLUSTRATION We report a case of a 54-year-old man who presented with headache, ideomotor apraxia, and gait disorder. A head computed tomography (CT) scan showed a biventricular hydrocephalus and a subsequent CT angiography documented the presence of a VBDE compressing the anterior part of the third ventricle that also appeared hypoplastic. The patient also presented a clinical history of arterial hypertension for which he was given a proper pharmacologic treatment with symptom relief. A surgical treatment of ventriculoperitoneal shunt along with endoscopic septostomy was proposed, but the patient refused, probably due to the slightly positive response to medical treatment. CONCLUSIONS: The natural clinical history of patients affected by VBDE is unfavorable with 7.8 years of median survival. The therapeutic strategy is usually conservative and the role of antiplatelets or oral anticoagulants is still debated. In selected patients, ventriculoperitoneal shunt to resolve intracranial hypertension caused by biventricular hydrocephalus is the most effective treatment. In our opinion, chronic third ventricle compression could lead to anatomic-pathologic alterations like the third ventricle hypoplasia documented in our report.